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Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency.

ABSTRACT: Protein C is one of the major inhibitors of the coagulation system that downregulate thrombin generation. Severe congenital protein C deficiency leads to a hypercoagulability state that usually presents at birth with purpura fulminans and/or severe venous and arterial thrombosis. Recurrent thrombotic events are commonly seen. From the 1990's, several virus-inactivated human protein C concentrates have been developed. These concentrates currently constitute the therapy of choice for the treatment and prevention of clinical manifestations of severe congenital protein C deficiency. This review summarizes the available information on the use of human protein C concentrates in patients with severe congenital protein C deficiency.

SUBMITTER: Kroiss S

PROVIDER: S-EPMC2846144 | biostudies-literature | 2010 Mar

REPOSITORIES: biostudies-literature

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Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency.

Kroiss Sabine S Albisetti Manuela M

Biologics : targets & therapy 20100324

Protein C is one of the major inhibitors of the coagulation system that downregulate thrombin generation. Severe congenital protein C deficiency leads to a hypercoagulability state that usually presents at birth with purpura fulminans and/or severe venous and arterial thrombosis. Recurrent thrombotic events are commonly seen. From the 1990's, several virus-inactivated human protein C concentrates have been developed. These concentrates currently constitute the therapy of choice for the treatment ...[more]

PMID: 20376174

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